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  1. A multisystem non caseous granulomatous disorder Q of unknown cause.
  2. It is a chronic multisystem disorder of unknown cause characterized by accumulation of T lymphocytes and phagocytes in various tissues of body
  3. Commonly affects adults aged 20-40yrs. 

Important Points: - Differential diagnosis of granulomatous disease

  1. Infections                     
    Bacteria: TB, Leprosy, Syphilis, Cat scratch fever
    Fungi: Cryptococcus neoformans, Coccidioides immitis
    Protozoa:  Schistosomiasis
  2. Autoimmune Primary biliary cirrhosis, Granulomatous orchitis
  3. Vasculitis   Giant cell arteritis, PAN, Takayasu’s arteritis
    Wegener’s granulomatosis
  4. Organic dust disease  Silicosis, Berylliosis
  5. Idiopathic  Crohn’s disease, de Quervain’s thyroiditis, Sarcoidosis
  6. Extrinsic allergic alveolitis 
  7. Histiocytosis X

Non caseating Sarcoid granulomas in affected organs

  1. Lung (90%)
    1. Interstitial lung disease
    2. Fibrosis of lung parenchyma
    3. Pleura is involved in 15% of cases U/L pleural effusion
    4. Cavitation is rare
  2. Lymph nodes (7-90%)
    1. B/L hilar lymphadenopathy is the hallmark of sarcoidosis
    2. Parotid enlargement B/L
  3. Skin
    1. Erythema nodosum
    2. Lupus pernio (purple blue shiny swollen lesions on nose, cheeks, lips, ears).
  4. Other
    1. Uveitis
    2. Hypercalcemia
    3. Peripheral neuropathy.
    4. Skeletal Arthritis
    5. Cor pulmonale

Clinical features of sarcoidosis Asymptomatic Q .

  1. Acute sarcoidosis often presents with erythema nodosum Q, polyarthralgia Q.
  2. Pulmonary disease 90% have abnormal CXRs with bilateral hilar lymphadenopathy Q (BHL), pulmonary infiltrates Q or fibrosis Q;
  3. Cavity formation and plural effusion are uncommon. 


  1. Dry cough, progressive dyspnea, exercise intolerance and chest pain.
  2. Non-pulmonary Q manifestations are lymphadenopathy; hepatomegaly, splenomegaly;
  3. Uveitis; conjunctivitis; keratoconjunctivitis sicca; glaucoma;
  4. Terminal phalangeal bone cysts.
  5. Enlargement of lacrimal and parotid glands
  6. Bell's palsy;(Seventh nerve is the most common cranial nerve involved) neuropathy; meningitis;
  7. Erythema Nodosum; lupus pernio; subcutaneous nodules;
  8. Cardiomyopathy; arrhythmias;
  9. Hypercalcemia; hypercalciuria; renal stones;
  10. Pituitary dysfunction.  (Note: All non pulmonary manifestations are MCQ) 

Important Points   Causes of BHL (bilateral hilar lymphadenopathy)

  1. Infection : TB
  2. Malignancy : Lymphoma, carcinoma, Mediastinal tumours
  3. Organic dust : Silicosis, Berylliosis
  4. Extrinsic allergic alveolitis
  5. Sarcoidosis      

Important Points: Inclusions seen in giant cells in sarcoidosis. (Remember as SARcoidosis)

  1. Schaumann bodies           
  2. Asteroid bodies    
  3. Residual bodies

Calcium Metabolism in Sarcoidosis (Ref. Hari. 18th ed., Pg -  2809) (AIIMS Nov 2010)

  1. Hypercalcemia and/or hypercalciuria occurs in about 10% of sarcoidosis patients.
  2. The mechanism of abnormal calcium metabolism is increased production of 1,25-dihydroxyvitamin D by the granuloma itself.
  3. The 1,25-dihydroxyvitamin D causes increased intestinal absorption of calcium, leading to hypercalcemia with a suppressed parathyroid hormone (PTH) level.




  1. ESR Increased                  
  2. Lymphopenia              
  3. Raised serum ACE  
  4. Raised serum Ca2+                  
  5. Raised immunoglobulins      
  6. Raised 24h urine Ca2+. 
Extra Edge:

ACE levels are increased in other granulomatous disease also.


Serum levels of ACE may be increased in:

  1. Sarcoidosis              
  2. Hypersensitivity pneumonitis       
  3. Asbestosis        
  4. Leprosy
  5. Silicosis                   
  6. Lymphoma                          
  7. Berylliosis            
  8. Tuberculosis
  9. Fungal infection        
  10. Granulomatous hepatitis 
  1. Bronchoalveolar lavage (BAL) shows increase lymphocytes in active disease, neutrophil with pulmonary fibrosis. A ratio of CD 4 to CD8 (CD4 / CD8) > 3.5 in BAL (But not in blood) is highly suggested of sarcoidoses.
  2. While there is marked enhancement of number of TH cells at the sites of disease, the number of TH cells in the blood are normal.

Extra Edge:


  1. Tuberculin skin test is negative
  2. CXR is abnormal in 90%:
    Stage 0: normal.
    Stage 1: B/L hilar lymph adrenopathy
    Stage 2: BHL + peripheral pulmonary infiltrate
    Stage 3: peripheral pulmonary infiltrates alone.
    Stage 4: progressive pulmonary fibrosis; bulla formation (honeycombing);

Extra Edge: Pleural effusion is Uncommon (MCQ)

  1. ECG may show arrhythmias or bundle branch block.
  2. Lung function tests impaired Q gas transfer, and a restrictive Lung Q defect
  3. Tissue biopsy (lung, liver, lymph nodes, skin nodules, or lacrimal glands) is diagnostic and shows non-caseating granulomata.
  4. Kveim test is positive.
  5. Ultrasound may show nephrocalcinosis or hepatosplenomegaly.
  6. Bone x-rays show 'punched out' lesions in terminal phalanges Q.
  7. CT/MRI may be useful in assessing severity of pulmonary disease or diagnosing neuro sarcoidosis.
  8. Ophthalmology assessment (slit lamp examination, fluorescein angiography) is indicated in ocular disease.
  9. A positive GALLIUM scan support diagnosis if increase activity is seen in parotid & lacrimal gland (PANDA sign) or right paratracheal and left hilar area (LAMBDA sign) 

Management: Patients with BHL alone do not require treatment since the majority recover spontaneously.

Acute sarcoidosis: 
: (Ref. Hari. 18th ed., Pg- 2813)

  1. NSAIDs.
  2. Hydroxychloroquine
  3. Indications for corticosteroid therapy:
    1. Parenchymal lung disease (symptomatic, static, or progressive)
    2. Uveitis
    3. Hypercalcemia
    4. Neurological or cardiac involvement
  4. Drugs used are: Prednisolone, IV methylprednisolone
  5. Immunosuppressants (methotrexate, cyclosporin, cyclophosphamide, Azathioprine) Infliximab

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